Liver transplantation in patients with cystic fibrosis: Analysis of united network for organ sharing data

Authors

  • Manuel Mendizabal,

    Corresponding author
    1. Unidad de Hígado y Trasplante Hepático, Hospital Universitario Austral, Pilar, Argentina
    2. Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA
    • Unidad de Hígado y Trasplante Hepático, Hospital Universitario Austral, Avenida Juan D. Perón 1500, Pilar, Argentina B1629AHJ
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    • Telephone: 54 2322 482884; FAX: 54 2322 482236

  • K. Rajender Reddy,

    1. Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA
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  • James Cassuto,

    1. Department of Surgery, Hospital of the University of Pennsylvania, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA
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  • Kim M. Olthoff,

    1. Department of Surgery, Hospital of the University of Pennsylvania, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA
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  • Thomas W. Faust,

    1. Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA
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  • George A. Makar,

    1. Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA
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  • Elizabeth B. Rand,

    1. Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA
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  • Abraham Shaked,

    1. Department of Surgery, Hospital of the University of Pennsylvania, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA
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  • Peter L. Abt

    1. Department of Surgery, Hospital of the University of Pennsylvania, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA
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  • This work was supported in part by the Health Resources and Services Administration (contract 231-00-0115). The content is the responsibility of the authors alone and does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the US Government.

Abstract

The improved life expectancy of patients with cystic fibrosis (CF) has led to a change in the impact of liver disease on the prognosis of this population. Liver transplantation has emerged as the procedure of choice for patients with CF and features of hepatic decompensation and for intractable variceal bleeding as a major manifestation. We retrospectively reviewed the United Network for Organ Sharing database to analyze the outcomes of 55 adults and 148 children with CF who underwent liver transplantation, and we compared them to patients who underwent transplantation for other etiologies. We additionally compared the benefits of liver transplantation among patients who underwent transplantation for cystic fibrosis–related liver disease (CFLD) and those who remained on the waiting list. The 5-year survival rates for children and adults undergoing liver transplantation were 85.8% and 72.7%, respectively (P = 0.016). A multivariate Cox regression analysis comparing pediatric and adult CF patients to patients who underwent transplantation for other etiologies noted lower 5-year survival rates (P < 0.0001). However, compared to those remaining on the waiting list, pediatric transplant recipients with CF (hazard ratio = 0.33, 95% confidence interval = 0.16-0.70, P = 0.004) and adult transplant recipients with CF (hazard ratio = 0.25, 95% confidence interval = 0.11-0.57, P = 0.001) gained a significant survival benefit. In conclusion, long-term outcomes in patients with CFLD are acceptable but are inferior in comparison with the outcomes of those undergoing transplantation for other etiologies. Despite such observations, a survival benefit was noted in transplant patients versus those who remained on the waiting list. Liver Transpl, 2011. © 2011 AASLD.

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